Pathology
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Fetopathology and developmental pathology of the embryo and fetus
Marta Ježová, Josef Feit et al.
Index
Contents
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+ Introduction
+ Using this atlas
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+ Support
+ Support
+ Atlas of fetal pathology
+ Congenital malformations
+ Physiological developement of an embryo and fetus, growth and changes of the external shape
+ Congenital malformations and congenital anomalies
+ Basic terminology
+ Etiology of congenital anomalies (CA)
+ Environmental causes of congenital anomalies, congenital infections
+ Rubella
+ Cytomegalovirus
+ Syphilis
+ Toxoplasmosis
+ Diabetes mellitus (DM) and pregnancy
+ Diabetic embryopathy
+ Diabetic fetopathy
+ Genetic causes of congenital malformations
+ Chromosomal abnormalities
+ Down syndrome
+ Klinefelter syndrome
+ Turner syndrome
+ Patau syndrome
+ Edwards syndrome
+ Triploidy
+ Single gene disorders
+ Tuberous sclerosis
+ Miscellaneous malformation syndromes, sequences and associations, unclassified
+ Sirenomelia (Mermaid syndrome)
+ Hydrops fetalis
+ Immune hydrops (erythroblastosis fetalis, hemolytic disease of the newborn)
+ Nonimmune hydrops
+ Hygroma colli cysticum
+ Pierre-Robin sequence
+ Caudal regression syndrome (sacral agenesis)
+ VACTERL association
+ Pathology of twinning
+ Complications of monochorionic diamnionic placentation
+ Chronic twin transfusion syndrome
+ Acute twin-twin transfusion
+ Complications of monochorionic monoamnionic placentation
+ Congenital malformations in twins
+ Chorangiopagus parasiticus (acardius)
+ Pathology of higher multiple gestations: triplets
+ Monsters
+ Monstra duplicia
+ Gemini monochoriati inequales
+ Duplicitas symmetros
+ Asymmetric dual monsters
+ Monstra simplicia
+ Congenital malformations of individual organ systems
+ Congenital malformations of the central nervous system
+ Congenital malformations of the spinal cord
+ Spina bifida
+ Myeloschisis (rachischisis posterior)
+ Congenital malformations of the brain
+ Anencephaly
+ Iniencephaly
+ Encephalocele
+ Arnold-Chiari malformation
+ Disorders of formation of the structures derived from the mediobasal prosencephalon
+ Migration disorders
+ Destructive lesions of fetal brain (disruptions)
+ Porencephaly
+ Hydranencephaly
+ Hydrocephalus
+ Dandy-Walker malformation
+ Intracranial non-neoplastic cysts
+ Arachnoid cyst
+ Neuroepithelial cyst
+ Congenital malformations of the respiratory system
+ Congenital cystic adenomatoid malformation (CCAM)
+ Lobar sequestration
+ Congenital lobar emphysema
+ Pulmonary hyperplasia
+ Pulmonary hypoplasia
+ Congenital pulmonary lymphangiectasia
+ Diaphragm and congenital malformations
+ Congenital malformations of kidneys and efferent urinary tracts
+ Renal agenesis
+ Variations of shape and position of the kidneys
+ Renal cystic disease
+ Infantile polycystic kidneys (ARKPD)
+ Renal dysplasia
+ Adult polycystic kidneys (ADPKD)
+ Autosomal dominant polycystic kidney disease in infant and fetus
+ Lower urinary tract obstruction
+ Posterior urethral valves
+ Congenital tubular renal dysgenesis
+ Urinary bladder
+ Hypospadia
+ Congenital malformations of genital system and somatosexual congenital disorders
+ Somatosexual disorders
+ Somatosexual disorders with normal karyotype
+ Pseudohermaphroditism masculinus
+ Androgen insensitivity syndrome, testicular feminization syndrome, female XY
+ Pseudohermaphroditism femininus
+ Congenital adrenal hyperplasia
+ Somatosexual disorders with pathologic karyotype
+ Congenital malformations of female genitalia
+ Congenital malformations of the internal genitalia
+ Congenital malformations associated with congenital malformations of urinary system
+ Congenital malformations of the vulva
+ Congenital malformations of the male genital organs
+ Cryptorchidism
+ Congenital malformations of penis and scrotum
+ Smith-Lemli-Opitz syndrome (SLOS)
+ Congenital tumors
+ Sacrococcygeal teratoma
+ Congenital cardiac rhabdomyoma
+ Congenital heart defects
+ Defects of heart septum
+ Atrial septal defects
+ Ventricular septal defects
+ AV septal defects
+ Anomalies of the great arteries
+ Transposition of the great arteries
+ Truncus arteriosus
+ Double-outlet right ventricle
+ Malformations of the valves
+ Tricuspid atresia
+ Stenosis and atresia of the mitral valve, the hypoplastic left heart syndrome
+ Stenosis of the pulmonary artery
+ Pulmonary atresia with intact ventricular septum
+ Tetralogy of Fallot
+ Aortic stenosis
+ Malformations of the aortic arch system
+ Persistence of ductus arteriosus
+ Coarctation of the aorta
+ Interruption of the aortic arch
+ Right-sided aortic arch
+ Malformations of the venous system
+ Anomalies of situs and heart position
+ Di George syndrome
+ Congenital malformations of the gastrointestinal tract
+ Oral cavity and the palate
+ Cleft lip and palate
+ Congenital defects of the esophagus
+ Atresia
+ Congenital esophagus stenoses
+ Congenital malformations of the stomach
+ Congenital malformations of the intestines
+ Atresia
+ Malrotation of the intestine
+ Remnants of the omphalomesentric duct
+ Anorectal malformations
+ Duplicatures
+ Defects of the abdominal wall
+ Omphalocele
+ Gastroschisis
+ Limb-body wall complex
+ Pentalogy of Cantrell
+ Developemental anomalies of the skeleton
+ Defects of growth of tubular bone and/or spine
+ Thanatophoric dysplasia
+ Diastrophic dysplasia
+ Abnormalities of density of cortical diaphyseal structure or metaphyseal modeling
+ Osteogenesis imperfecta
+ Limb malformations
+ Terminal transverse limb defects
+ Radial ray defects
+ Finger anomalies
+ Club foot
+ Constriction (amniotic) band syndrome
+ Placental inflammation
+ Ascending infections
+ Placental inflammation, acute
+ Hematogenous infections
+ Fetomaternal listeriosis
+ Pathology of the fetal membranes
+ Amniotic band syndrome
+ Meconium staining
+ Candida infection of the placenta
+ Pathology of the placenta
+ Normal placenta
+ Extrauterine gravidity
+ Gestational trophoblastic disease (GTD)
+ Hydatidiform mole
+ Complete mole
+ Partial mole
+ Invasive mola (mole proliferans, mole destruens)
+ Gestational choriocarcinoma
+ Placental site trophoblastic tumor
+ Placenta and major chromosomal abnormalities
+ Hydropic placenta
+ Pathology of the umbilical cord
+ Abnormal length
+ Short cord
+ Long cord
+ Insertion abnormalities
+ Abnormal coiling
+ Umbilical vessels pathology
+ Umbilical cord accidents which compromise the blood flow
+ Thrombosis of umbilical blood vessels
+ Umbilical cord inflammation
+ Acute funisitis
+ Subacute necrotizing funisitis
+ Miscellaneous rare cord lesions
Duplicitas symmetros
Introduction

Twins are symmetric and relatively well developed, although anomalies are also frequent. Ther are two types of symmetric monsters: disymmetric (planes of symmetry of both parts are at right angles to each other) and monosymmetric.

The difference is that disymetric thoracopagic twins look at each other, while monosymetric twins both look in the same direction.

The possibility for successful surgical separation of such twins depends on the organs shared and other congenital malformations.

Examples: Siamese twins (Chang and Eng Bunker who were xiphopagous) or the Czech Blazek sisters (monosymetric pygopagus).

Classification
  • Thoracal conjunctions:
    • xiphopagus
    • sternopagus
    • thoracopagus
      • disymmetric form: partially shared trunk, look at each other
      • monosymmetric form: shoulders are fused at angle, arms on the back side may be fused (tribrachius)
      • monosymmetric form has a better prognosis
      • classification according to heart sharing:
        • separate hearts, each in its own pericardium
        • separate hearts sharing one pericardium
        • one heart with atrial union
        • one heart with both atrial and ventricular union
  • Conjunctions involving the head and trunk:
    • prosopothoracopagus
    • cephalothoracopagus
  • Conjunctions involving the pelvis and abdomen:
    • ileoxiphopagus
    • ileothoracopagus
    • pygopagus (dorsal “fusion”)
  • Monsters with a horizontal axis of symmetry:
    • ischiopagus
    • craniopagus
    • diprosopos
    • dicephalus
Case study
Thoracopagus
Marta Ježová
History
  • 11th pregnancy, 7th delivery
  • the mother has not been examined during the pregnancy
  • premature, precipitous labour of the twins
  • vaginal delivery
Macroscopic appearance
  • male thoracopagus
  • numerous minor external anomalies and internal organ malformations, some of them shared, the others only in twin A:
    • preaxial polydaktyly
    • hypospadia
    • anomalous gothic palate and hypoplasia of the maxila of the fetus A
    • omphalocele
    • esophageal atresia and tracheoesophageal fistula in both fetuses
  • sharing of internal organs:
    • shared liver
    • partially shared intestines — duodenum and small intestine until the omphaloenteric duct
    • shared pericardium
    • shared heart in the shape of a wide letter U, externally “fused in the apex region”
  • the heart:
    • shared atrium
    • shared ventricles via two muscular defects
      • univentricular heart: left ventricle belongs to the fetus A, right ventricle to the fetus B
      • functionally 4 compartments and 2 rudimental outlet chambers
      • arterial trasposition in fetus A, pulmonary arteries are hypoplastic
      • anomalies of systemic and pulmonary venous return

Thoracopagus (72276)

Thoracopagus, connecting bridge (72289)

Pictures

Thoracopagus, whole view: Thoracopagus, Macro, autopsy (72276) Thoracopagus, Macro, autopsy (72288) Thoracopagus, Macro, autopsy (72287)

Omphalocele: Thoracopagus, Macro, autopsy (72278)

Upper limb malformation: Thoracopagus, Macro, autopsy (72281)

Hypospadia: Thoracopagus, Macro, autopsy (72282)

Missing philtrum, twin A: Thoracopagus, Macro, autopsy (72283)

Twin B: Thoracopagus, Macro, autopsy (72286)

Thoracal conjunction, connecting bridge (shared liver, shared malformed heart): Thoracopagus, Macro, autopsy (72289) Thoracopagus, Macro, autopsy (72290) Thoracopagus, Macro, autopsy (72291) Thoracopagus, Macro, autopsy (72292)

Scheme of the malformed heart: Thoracopagus, scheme of the heart, Macro, autopsy (72303)

Liver, intestine: Thoracopagus, Macro, autopsy (72295) Thoracopagus, Macro, autopsy (72296) Thoracopagus, Macro, autopsy (72297)